Introduction:
Extraovarian primary peritoneal carcinoma (EOPPC) is a relatively new disease that develops only in women. In the disease, primarily abdominal carcinoma is seen. It minimally or does not involve ovaries. Most cases involve serous cells. EOPPC is different from serous ovarian cancer but resembles it in terms of clinical presentation, microscopic morphology, and response to chemotherapy. EOPPC develops from the peritoneum lining that covers the pelvis and abdomen. It contains approximately ten percent of all cases considered with a presumed diagnosis of ovarian cancer. The prognosis of such individuals suffering from EOPPC is often poor. Median life expectancy is one to two years.
It can give rise to a different carcinoma, such as:
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Serous surface papillary carcinoma.
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Primary peritoneal carcinoma.
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Peritoneal mesothelioma.
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Multiple focal extra ovarian serous carcinoma.
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Primary peritoneal papillary serous adenocarcinoma.
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Serous surface carcinoma of the peritoneum.
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Extraovarian peritoneal serous papillary carcinoma.
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Extraovarian Mullerian adenocarcinoma.
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Normal-sized ovary carcinoma syndrome.
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Papillary serous carcinoma of the peritoneum.
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Peritoneal papillary carcinoma.
What Is the History Behind Extraperitoneal Peritoneal Carcinoma?
Swerdlow reported the first case in 1959 in a 27-year woman with pelvic pain and an adnexal mass. Further examination revealed the presence of a friable pelvic tumor, but fallopian tubes and ovaries were normal.
What Is the Pathophysiology of Extraperitoneal Peritoneal Carcinoma?
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There are two thoughts of school in the explanation of this tumor. First, believe that some embryonic germ cells are on the gonadal embryonic pathway. In some cases, these cells undergo a malignant or cancerous transformation that leads to the development of EOPPC.
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The second thought believes that cancer originates from the epithelium lining of the abdominal cavity (peritoneum) and the ovaries (germinal epithelium) due to some cancerous stimulus.
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And also, mutations or alterations in the p53 gene are seen and considered a responsible factor. In some cases, mutation of the BRCA1 gene is also seen.
What Is the Incidence of Extraperitoneal Peritoneal Carcinoma?
EOPCC is a rare entity. A relatively small number of cases are reported because EOPPC is a relatively newly defined disease entity, and in most cases, EOPPC is misdiagnosed as ovarian cancer.
What Are the Risk Factors Associated With Extraperitoneal Peritoneal Carcinoma?
The risk factors for EOPPC are unknown. But there are some factors that are associated with increased risk for EOPPC.
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Increased Age: Women after 50 are at greater risk than others.
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Menopause: Women, after menopause or cessation of their menstrual cycle, are at greater risk than others, and women who use hormone replacement therapy are at greater risk.
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Smoking: The risk of development is more in smokers than non-smokers.
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Birth Control Pill Use: Abrupt and unprescribed frequent use of birth pills increased the risk of developing EOPPC.
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Obesity: Women with higher amounts of body fat are at a risk.
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Family History: A family history of ovarian cancer is also a risk factor that can lead to EOPPC.
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Infertility: Infertility can be a contributing factor in the development of EOPPC. Women with a history of infertility and no biological children are at significant risk.
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Breast Cancer: Personal history of breast cancer or uterine cancer.
What Are the Clinical Features Associated With Extraperitoneal Peritoneal Carcinoma?
The EOPPC is primarily seen in white women, mostly in middle age.
The common symptoms include
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The most common presenting feature is ascites (fluid accumulation in the abdomen).
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Distention in abdomen.
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Pain in the abdomen.
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Gastrointestinal symptoms such as nausea, vomiting, and dyspepsia (burning sensation).
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Change in bowel habits.
On examination, an intraperitoneal tumor is usually seen in the upper abdomen, and no ovarian involvement. Because the ovaries do not show abnormality, EOPPC is also known as normal-sized ovary carcinoma syndrome. Extraovarian primary peritoneal carcinoma can spread to other areas, such as lymph nodes, liver parenchyma, and the brain. In addition, it can spread through lymphatics or blood. The elevation of tumor marker CA 125 is seen in most of the patients.
What Are the Diagnostic Criteria for Extraperitoneal Peritoneal Carcinoma?
To confirm the presence of EOPPC following requirements should be met:
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Both ovaries must be standard size (not more than four centimeters).
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A tumor present at the extra ovarian sites should be greater than the surface of either ovary.
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Microscopically, also the ovarian component must be normal.
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Histologic and cytologic characteristics of the tumor must be predominantly of the serous type that is similar or identical to any grade of ovarian serous papillary adenocarcinoma.
What Is the Management for Extraperitoneal Peritoneal Carcinoma?
The management of EOPPC is similar to epithelial ovarian cancer. Treatment consists of surgical removal followed by the drug Cisplatin (Platinol) chemotherapy. The success of treatment with primary surgery in EOPPC patients is limited. Treatment with Cisplatin is the most common first-line chemotherapeutic regimen used in patients of EOPPC.
What Is the Survival Rate for Extraperitoneal Peritoneal Carcinoma?
The disease has poor survival in most patients. After treatment with surgery, survival can be improved, followed by the combination of Cisplatin and Doxorubicin with or without Cyclophosphamide.
What Are the Prognostic Factors Associated with Extraperitoneal Peritoneal Carcinoma?
Knowledge of prognostic factors in EOPPC patients is limited. Dividing tumors (mitosis) have a worse prognosis than non-dividing tumors. The prognosis is good for patients who receive multiple-agent chemotherapy compared to single-agent chemotherapy. The prognosis is good for those who received Cisplatin compared to non-Cisplatin-containing regimens.
Grade of the tumor, histology, ovarian involvement, elevated CA 125 values, and p53 overexpression do not affect survival significantly. However, patients with smaller tumor volumes and less aggressive tumors are more likely to have a good prognosis after cytoreduction (surgical reduction).
Conclusion:
EOPPC is a relatively new entity. Its occurrence is rare. Tumor mainly affects peritoneal cavity lining other than ovaries. Treatment of the condition is somehow similar to ovarian carcinoma. It can spread to other body regions but there is no metastatic involvement of the ovary. White females of middle age are at significant risk than others. The prognosis is not very good for the condition. But advanced treatment modality increased the survival rate in patients. Surgical removal, along with Cisplatin medication, gives the best possible result.
